336 related articles for article (PubMed ID: 32630527)
1. Full Rescue of F508del-CFTR Processing and Function by CFTR Modulators Can Be Achieved by Removal of Two Regulatory Regions.
Uliyakina I; Botelho HM; da Paula AC; Afonso S; Lobo MJ; Felício V; Farinha CM; Amaral MD
Int J Mol Sci; 2020 Jun; 21(12):. PubMed ID: 32630527
[TBL] [Abstract][Full Text] [Related]
2. Two Small Molecules Restore Stability to a Subpopulation of the Cystic Fibrosis Transmembrane Conductance Regulator with the Predominant Disease-causing Mutation.
Meng X; Wang Y; Wang X; Wrennall JA; Rimington TL; Li H; Cai Z; Ford RC; Sheppard DN
J Biol Chem; 2017 Mar; 292(9):3706-3719. PubMed ID: 28087700
[TBL] [Abstract][Full Text] [Related]
3. Lipophilicity of the Cystic Fibrosis Drug, Ivacaftor (VX-770), and Its Destabilizing Effect on the Major CF-causing Mutation: F508del.
Chin S; Hung M; Won A; Wu YS; Ahmadi S; Yang D; Elmallah S; Toutah K; Hamilton CM; Young RN; Viirre RD; Yip CM; Bear CE
Mol Pharmacol; 2018 Aug; 94(2):917-925. PubMed ID: 29903751
[TBL] [Abstract][Full Text] [Related]
4. Restoration of R117H CFTR folding and function in human airway cells through combination treatment with VX-809 and VX-770.
Gentzsch M; Ren HY; Houck SA; Quinney NL; Cholon DM; Sopha P; Chaudhry IG; Das J; Dokholyan NV; Randell SH; Cyr DM
Am J Physiol Lung Cell Mol Physiol; 2016 Sep; 311(3):L550-9. PubMed ID: 27402691
[TBL] [Abstract][Full Text] [Related]
5. Characterization of the mechanism of action of RDR01752, a novel corrector of F508del-CFTR.
Lopes-Pacheco M; Silva IAL; Turner MJ; Carlile GW; Sondo E; Thomas DY; Pedemonte N; Hanrahan JW; Amaral MD
Biochem Pharmacol; 2020 Oct; 180():114133. PubMed ID: 32628927
[TBL] [Abstract][Full Text] [Related]
6. ORKAMBI-Mediated Rescue of Mucociliary Clearance in Cystic Fibrosis Primary Respiratory Cultures Is Enhanced by Arginine Uptake, Arginase Inhibition, and Promotion of Nitric Oxide Signaling to the Cystic Fibrosis Transmembrane Conductance Regulator Channel.
Wu YS; Jiang J; Ahmadi S; Lew A; Laselva O; Xia S; Bartlett C; Ip W; Wellhauser L; Ouyang H; Gonska T; Moraes TJ; Bear CE
Mol Pharmacol; 2019 Oct; 96(4):515-525. PubMed ID: 31427400
[TBL] [Abstract][Full Text] [Related]
7. Low free drug concentration prevents inhibition of F508del CFTR functional expression by the potentiator VX-770 (ivacaftor).
Matthes E; Goepp J; Carlile GW; Luo Y; Dejgaard K; Billet A; Robert R; Thomas DY; Hanrahan JW
Br J Pharmacol; 2016 Feb; 173(3):459-70. PubMed ID: 26492939
[TBL] [Abstract][Full Text] [Related]
8. Lumacaftor alone and combined with ivacaftor: preclinical and clinical trial experience of F508del CFTR correction.
Brewington JJ; McPhail GL; Clancy JP
Expert Rev Respir Med; 2016; 10(1):5-17. PubMed ID: 26581802
[TBL] [Abstract][Full Text] [Related]
9. Phosphorylation of the Chaperone-Like HspB5 Rescues Trafficking and Function of F508del-CFTR.
Degrugillier F; Aissat A; Prulière-Escabasse V; Bizard L; Simonneau B; Decrouy X; Jiang C; Rotin D; Fanen P; Simon S
Int J Mol Sci; 2020 Jul; 21(14):. PubMed ID: 32650630
[TBL] [Abstract][Full Text] [Related]
10. Extent of rescue of F508del-CFTR function by VX-809 and VX-770 in human nasal epithelial cells correlates with SNP rs7512462 in SLC26A9 gene in F508del/F508del Cystic Fibrosis patients.
Kmit A; Marson FAL; Pereira SV; Vinagre AM; Leite GS; Servidoni MF; Ribeiro JD; Ribeiro AF; Bertuzzo CS; Amaral MD
Biochim Biophys Acta Mol Basis Dis; 2019 Jun; 1865(6):1323-1331. PubMed ID: 30716472
[TBL] [Abstract][Full Text] [Related]
11. In utero and postnatal ivacaftor/lumacaftor therapy rescues multiorgan disease in CFTR-F508del ferrets.
Evans IA; Sun X; Liang B; Vegter AR; Guo L; Lynch TJ; Zhang Y; Zhang Y; Yi Y; Yang Y; Feng Z; Park SY; Shonka A; McCumber H; Qi L; Wu P; Liu G; Lacina A; Wang K; Gibson-Corley KN; Meyerholz DK; Limoli DH; Rosen BH; Yan Z; Bartels DJ; Engelhardt JF
JCI Insight; 2024 Apr; 9(8):. PubMed ID: 38646935
[TBL] [Abstract][Full Text] [Related]
12. Differential thermostability and response to cystic fibrosis transmembrane conductance regulator potentiators of human and mouse F508del-CFTR.
Bose SJ; Bijvelds MJC; Wang Y; Liu J; Cai Z; Bot AGM; de Jonge HR; Sheppard DN
Am J Physiol Lung Cell Mol Physiol; 2019 Jul; 317(1):L71-L86. PubMed ID: 30969810
[TBL] [Abstract][Full Text] [Related]
13. The investigational Cystic Fibrosis drug Trimethylangelicin directly modulates CFTR by stabilizing the first membrane-spanning domain.
Laselva O; Molinski S; Casavola V; Bear CE
Biochem Pharmacol; 2016 Nov; 119():85-92. PubMed ID: 27614011
[TBL] [Abstract][Full Text] [Related]
14. Measurements of Functional Responses in Human Primary Lung Cells as a Basis for Personalized Therapy for Cystic Fibrosis.
Awatade NT; Uliyakina I; Farinha CM; Clarke LA; Mendes K; Solé A; Pastor J; Ramos MM; Amaral MD
EBioMedicine; 2015; 2(2):147-53. PubMed ID: 26137539
[TBL] [Abstract][Full Text] [Related]
15. Unravelling the Regions of Mutant F508del-CFTR More Susceptible to the Action of Four Cystic Fibrosis Correctors.
Amico G; Brandas C; Moran O; Baroni D
Int J Mol Sci; 2019 Nov; 20(21):. PubMed ID: 31683989
[TBL] [Abstract][Full Text] [Related]
16. Cystic fibrosis transmembrane conductance regulator-modifying medications: the future of cystic fibrosis treatment.
Pettit RS
Ann Pharmacother; 2012; 46(7-8):1065-75. PubMed ID: 22739718
[TBL] [Abstract][Full Text] [Related]
17. VX-809 corrects folding defects in cystic fibrosis transmembrane conductance regulator protein through action on membrane-spanning domain 1.
Ren HY; Grove DE; De La Rosa O; Houck SA; Sopha P; Van Goor F; Hoffman BJ; Cyr DM
Mol Biol Cell; 2013 Oct; 24(19):3016-24. PubMed ID: 23924900
[TBL] [Abstract][Full Text] [Related]
18. Identification of novel F508del-CFTR traffic correctors among triazole derivatives.
Bacalhau M; Ferreira FC; Kmit A; Souza FR; da Silva VD; Pimentel AS; Amaral MD; Buarque CD; Lopes-Pacheco M
Eur J Pharmacol; 2023 Jan; 938():175396. PubMed ID: 36410419
[TBL] [Abstract][Full Text] [Related]
19. F508del-cystic fibrosis transmembrane regulator correctors for treatment of cystic fibrosis: a patent review.
Yang H; Ma T
Expert Opin Ther Pat; 2015; 25(9):991-1002. PubMed ID: 25971311
[TBL] [Abstract][Full Text] [Related]
20. Correctors (specific therapies for class II CFTR mutations) for cystic fibrosis.
Southern KW; Patel S; Sinha IP; Nevitt SJ
Cochrane Database Syst Rev; 2018 Aug; 8(8):CD010966. PubMed ID: 30070364
[TBL] [Abstract][Full Text] [Related]
[Next] [New Search]
